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Complement sensitivity of paroxysmal nocturnal hemoglobinuria bone marrow
cells
J Tumen, LB Kline, JW Fay, DC Scullin, EG Reisner, WF Rosse and AT Huang
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder in which
erythrocytes, granulocytes, and platelets are defective, as shown by
increased susceptibility of RBCs, WBCs, and platelets to complement-
mediated lysis in vitro. The purpose of this study is to determine the
sensitivity to complement lysis of PNH and non-PNH erythroid and myeloid
precursors using the release of 59Fe and myeloperoxidase as specific
markers to monitor the lytic action of complement on erythroid and myeloid
cell precursors, respectively. Erythroid cell precursors in four of four
PNH patients demonstrated increased sensitivity to complement-mediated
lysis. Myeloid cell precursors in four of five PNH patients also exhibited
increased sensitivity to complement and antibody. In addition, CFU-c growth
was below normal in the marrow of seven PNH patients. These findings
support the hypothesis that the defect in PNH occurs at the level of the
hematopoietic stem cell.
Volume 55,
Issue 6,
pp. 1040-1046,
06/01/1980
Copyright © 1980 by The American Society of Hematology
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